Alport Syndrome is a hereditary from of progressive renal disease characterized by bloody urine (hematuria) and progressive renal failure and associated with hearing loss and eye abnormalities. Affected males all eventually progress to end stage renal failure while in affected females renal failure is delayed until later in life or does not occur. The genetic defect in most families results in the failure to produce and/or incorporate certain collagen chains into basement membranes of the body. This study utilizes new electron microscopic techniques in order to clarify the relationship of the missing collagen chains to the structural and functional manifestations of Alport Syndrome.